Surgical outcomes for pediatric congenital lung malformation: 13 years' experience

Abstract

Background: Congenital lung malformations (CLM) are a gamut of lesions that originate throughout the embryonic period and manifest in the neonatal or sporadically in the prenatal period, characteristically might stay well for some time, to be found inadvertently or to present with complications. In the 13 years from Jan. 2003 to Dec. 2015, this prospective cohort study included consecutive pediatric patients under 12 years old, who presented either emergently or electively with any variety of CLM. The lesions encompassed in this assortment were congenital lobar emphysema (CLE), congenital pulmonary airway malformation (CPAM), bronchogenic cysts (BC), and bronchopulmonary sequestration (BPS).

Results: Sixty-eight pediatric patients with CLM were operated at our institution; 18 CPAMs, 22 CLEs, 19 BPSs (17 intralobar and 2 extralobar), and 9 patients with BCs. The patients' age ranged from 1 to 54 months (mean age of 10.73 ± 9.73 months), with overall male gender predominance (61.76%). Both CLE and CPAM had a male predominance, while BC and BPS had equivocal gender distribution. CLE patients had the earliest presentation at 2.89 ± 1.5 months and congenital cystic adenomatoid malformations (CCAM) had the latest presentation at 21.78 ± 15.6 months (F = 15.27, p < 0.0001). Lobectomy was the commonest procedure performed. Fifty-nine lobectomies were performed (21 LUL, 15 RLL, 14 LLL, 8 RUL, and 1 middle lobectomy). Six cystectomies were performed for BC. Twenty-three cases (33.8%) had postoperative complications that were mainly significant or prolonged air leak (13.24%), pneumonia (5.88%), 3 cases of hemothorax (4.4%), pulmonary atelectasis in 2 patients (2.94%), 1 patient developed effusion (1.47%), and there were 2 mortalities.

Conclusions: CLM must be in mind in the differential diagnoses of any case with repeated infection, respiratory distress, or radiological abnormalities. Surgery in the form of lobectomy or lesser resection is generally safe.

Keywords: Lung malformations; Lung volume reduction surgery; Pediatric lung disease; Thoracic surgery.

Fig. 1

Congenital cystic adenomatoid malformation (CPAM). a Specimen of right upper lobectomy for CPAM. b Low power light microscopy for Hematoxylin and Eosin film for the excised specimen exhibiting characteristic sawtooth lining with pseudostratified epithelium and mucous cells, consistent with type 1 CPAM. c Saggital computed chest tomography cut showing a large cyst of the case. d Computed tomography coronal cut for the same case

Fig. 2

Proposed algorithm by Annunziata et al. for follow-up of congenital lung malformations. [26]